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Free access | 10.1172/JCI106725
Department of Medicine, University of Toronto, Toronto 181, Canada
Banting and Best Department of Medical Research, University of Toronto, Toronto 181, Canada
Find articles by Halperin, M. in: JCI | PubMed | Google Scholar
Department of Medicine, University of Toronto, Toronto 181, Canada
Banting and Best Department of Medical Research, University of Toronto, Toronto 181, Canada
Find articles by Schiller, C. in: JCI | PubMed | Google Scholar
Department of Medicine, University of Toronto, Toronto 181, Canada
Banting and Best Department of Medical Research, University of Toronto, Toronto 181, Canada
Find articles by Fritz, I. in: JCI | PubMed | Google Scholar
Published November 1, 1971 - More info
We report the effects of methylmalonic acid (MMA) on the mitochondrial transport systems for malate, α-oxoglutarate, and isocitrate. MMA is shown to be a substrate for all three carrier systems, and an inhibitor of the malate-phosphate exchange carrier. The effects of MMA on the metabolism of malate, oxoglutarate, and isocitrate by rat liver mitochondria are demonstrated to be mediated by the influence of MMA on the transport step. A hypothesis regarding the metabolic impairments responsible for hypoglycemia and ketonemia in methylmalonic aciduria is formulated in relation to these findings.